Late onset and rapidly progressive geographic macular atrophy starting in the fifth decade, associated with pseudo-drusen or reticular drusen in the posterior pole and paving stone degeneration in the retinal periphery.
September 8, 2011
A New Clinical Entity
In 2000 we became interested in this peculiar retinal dystrophy. We saw a lady with a jugum meningioma complaining of malaise and vertigo. She presented a severe central arolar atrophy in both eyes with pseudo-drusen and peripheral degenerative lesions. The meningioma was unrelated to her fundus condition. We were intrigued by this condition and since then collected about ten cases with this triple association of central geographic atrophy, macular small hard drusen and pseudodrusen (reticular drusen) and peripheral paving stone degeneration and compared and published them with cases of with similar appearance originating from other regions ( .http://www.ncbi.nlm.nih.gov/pubmed/19181301 ). The lesions affect both eyes symmetrically; they tend to progress over a number of years to a major central areolar atrophy with loss of central vision and progressive involvement of the electroretinogram.